Pleuropulmonary Blastoma: a Clinicopathological Analysis
Background and objective Pleuropulmonary blastoma (PPB) is a rare malignant tumor with unique clinicopathological features. The aim of this study is to investigate the clinicopathological features, the diagnosis and differential diagnosis of pleuropulmonary blastoma. Methods Five cases of PPB were a...
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| Format: | Article |
| Language: | zho |
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Chinese Anti-Cancer Association; Chinese Antituberculosis Association
2010-05-01
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| Series: | Chinese Journal of Lung Cancer |
| Subjects: | |
| Online Access: | http://www.lungca.org/index.php?journal=01&page=article&op=viewFile&path[]=10.3779%2Fj.issn.1009-3419.2010.05.31&path[]=1497 |
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| author | Jun GAO Shangfu ZHANG Shifeng WANG Shigao CHEN |
| author_facet | Jun GAO Shangfu ZHANG Shifeng WANG Shigao CHEN |
| author_sort | Jun GAO |
| collection | DOAJ |
| description | Background and objective Pleuropulmonary blastoma (PPB) is a rare malignant tumor with unique clinicopathological features. The aim of this study is to investigate the clinicopathological features, the diagnosis and differential diagnosis of pleuropulmonary blastoma. Methods Five cases of PPB were analyzed by light microscopy, immunohistochemistry and their clinical data, and the relative literatures were reviewed. Results Five cases of patients suffered from PPB were aged from 21 to 47 months (mean 32.8 months). Most of the masses were located in the thoracic cavities and 4 cases accompanied with pleural effusions. Histologically, these tumors included 1 case of type I PPB which showed pure cystic architecture; 2 cases were type II PPB which showed cystic and solid masses accompanied with rhabdomyoblastic differentiation and nodules of cartilage; the other 2 cases were type III PPB and characterized by absolute solid masses with anaplastic undifferentiated sarcomatous components. Immunohistochemical studies showed that tumor cells were positive for Vimentin and some for Desmin and Myogenin, the nodules of cartilage were positive for S-100. The tumor cells were negative for PCK, EMA and CD99. Conclusion Pleuropulmonary blastoma is a rare and highly aggressive malignancy arising in the lung and pleural of infancy and early childhood. The type I, II and III PPB have unique clinicopathological features respectively. This kind of tumor should be distinguished from some benign and malignant diseases such as congenital cystic adenomatoid malformation (CCAM) and embryonal rhabdomyosarcoma. |
| first_indexed | 2024-12-13T09:33:58Z |
| format | Article |
| id | doaj.art-adb29dd45633466d91dfc02feb036157 |
| institution | Directory Open Access Journal |
| issn | 1009-3419 1999-6187 |
| language | zho |
| last_indexed | 2024-12-13T09:33:58Z |
| publishDate | 2010-05-01 |
| publisher | Chinese Anti-Cancer Association; Chinese Antituberculosis Association |
| record_format | Article |
| series | Chinese Journal of Lung Cancer |
| spelling | doaj.art-adb29dd45633466d91dfc02feb0361572022-12-21T23:52:24ZzhoChinese Anti-Cancer Association; Chinese Antituberculosis AssociationChinese Journal of Lung Cancer1009-34191999-61872010-05-01135550553Pleuropulmonary Blastoma: a Clinicopathological AnalysisJun GAOShangfu ZHANGShifeng WANGShigao CHENBackground and objective Pleuropulmonary blastoma (PPB) is a rare malignant tumor with unique clinicopathological features. The aim of this study is to investigate the clinicopathological features, the diagnosis and differential diagnosis of pleuropulmonary blastoma. Methods Five cases of PPB were analyzed by light microscopy, immunohistochemistry and their clinical data, and the relative literatures were reviewed. Results Five cases of patients suffered from PPB were aged from 21 to 47 months (mean 32.8 months). Most of the masses were located in the thoracic cavities and 4 cases accompanied with pleural effusions. Histologically, these tumors included 1 case of type I PPB which showed pure cystic architecture; 2 cases were type II PPB which showed cystic and solid masses accompanied with rhabdomyoblastic differentiation and nodules of cartilage; the other 2 cases were type III PPB and characterized by absolute solid masses with anaplastic undifferentiated sarcomatous components. Immunohistochemical studies showed that tumor cells were positive for Vimentin and some for Desmin and Myogenin, the nodules of cartilage were positive for S-100. The tumor cells were negative for PCK, EMA and CD99. Conclusion Pleuropulmonary blastoma is a rare and highly aggressive malignancy arising in the lung and pleural of infancy and early childhood. The type I, II and III PPB have unique clinicopathological features respectively. This kind of tumor should be distinguished from some benign and malignant diseases such as congenital cystic adenomatoid malformation (CCAM) and embryonal rhabdomyosarcoma.http://www.lungca.org/index.php?journal=01&page=article&op=viewFile&path[]=10.3779%2Fj.issn.1009-3419.2010.05.31&path[]=1497Pleuropulmonary blastomaImmunohistochemistryPathologic diagnosisDifferential diagnosis |
| spellingShingle | Jun GAO Shangfu ZHANG Shifeng WANG Shigao CHEN Pleuropulmonary Blastoma: a Clinicopathological Analysis Chinese Journal of Lung Cancer Pleuropulmonary blastoma Immunohistochemistry Pathologic diagnosis Differential diagnosis |
| title | Pleuropulmonary Blastoma: a Clinicopathological Analysis |
| title_full | Pleuropulmonary Blastoma: a Clinicopathological Analysis |
| title_fullStr | Pleuropulmonary Blastoma: a Clinicopathological Analysis |
| title_full_unstemmed | Pleuropulmonary Blastoma: a Clinicopathological Analysis |
| title_short | Pleuropulmonary Blastoma: a Clinicopathological Analysis |
| title_sort | pleuropulmonary blastoma a clinicopathological analysis |
| topic | Pleuropulmonary blastoma Immunohistochemistry Pathologic diagnosis Differential diagnosis |
| url | http://www.lungca.org/index.php?journal=01&page=article&op=viewFile&path[]=10.3779%2Fj.issn.1009-3419.2010.05.31&path[]=1497 |
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